An interrupted aortic arch is a rare heart defect. The aortic arch is part of the major blood vessel that helps move blood from the heart to the rest of the body. With this defect, the aortic arch is interrupted or incomplete. Blood cannot flow through it normally. This makes blood flow to the body less efficient. Children with this defect may also have a hole in the wall between the right and left chambers in the heart.
An interrupted aortic arch is present at birth. A direct cause is not known. The defect develops in the fifth to seventh week of fetal growth.
There is an increased risk for this condition if your child also has DiGeorge syndrome. This is a chromosomal abnormality.
Symptoms typically appear within the first day or two after birth. Many times, the baby will show symptoms soon after birth. Tell your doctor if you notice the following in your infant or child:
This condition can lead to shock and heart failure. Your child will need emergency care.
During the exam, the doctor may detect:
These symptoms may be due to other conditions.
The doctor will ask about your child's symptoms and medical history. A physical exam will be done.
Images may be taken of your child's chest. This can be done with:
Your child's heart activity may be measured. This can be done with electrocardiogram (EKG).
Talk with the doctor about the best treatment plan for your child. Treatment options include:
Certain medications will usually be given to keep some blood flowing through another blood vessel called the ductus arteriosus. This allows some blood to get around the interruption in the aorta. This is a temporary treatment.
Medications may also:
Surgery is needed to correct the defect. Surgery aims to form a connection between the two parts of the aortic arch. The hole in the heart between the ventricles is also closed. The ductus arteriosus is then closed.
American Heart Association
Healthy Children—American Academy of Pediatrics
Canadian Cardiovascular Society
Heart and Stroke Foundation of Canada
Interrupted aortic arch. Cincinnati Children’s Hospital Medical Center website. Available at: http://www.cincinnatichildrens.org/health/i/iaa. Updated November 2012. Accessed July 17, 2013.
Interrupted aortic arch. Johns Hopkins Children's Center website. Available at: http://www.hopkinschildrens.org/Interrupted-Aortic-Arch.aspx. Updated May 16, 2011. Accessed July 17, 2013.
Last reviewed June 2015 by Michael Woods, MD
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.